miércoles, 21 de agosto de 2019

Unusual Cancers of Childhood Treatment (PDQ®) 5/5 –Patient Version - National Cancer Institute

Unusual Cancers of Childhood Treatment (PDQ®)–Patient Version - National Cancer Institute

National Cancer Institute

Unusual Cancers of Childhood Treatment (PDQ®)–Patient Version

Other Rare Unusual Cancers of Childhood

Multiple Endocrine Neoplasia Syndromes

Multiple endocrine neoplasia (MEN) syndromes are inherited disorders that affect the endocrine system. The endocrine system is made up of glands and cells that make hormones and release them into the blood. MEN syndromes may cause hyperplasia (the growth of too many normal cells) or tumors that may be benign (not cancer) or malignant(cancer).
There are several types of MEN syndromes and each type may cause different conditionsor cancers. A mutation in the RET gene is usually linked to medullary thyroid cancer in MEN2 syndrome. If a diagnosis of MEN2 syndrome is suspected for the child or a family member is diagnosed with MEN2 syndrome, the parents should receive genetic counselingbefore genetic testing is done for the child. Genetic counseling also includes a discussion of the risk of MEN2 syndrome for the child and other family members.
The two main types of MEN syndromes are MEN1 and MEN2:
  • MEN1 syndrome is also called Wermer syndrome. This syndrome usually causes tumors in the parathyroid gland, pituitary gland, or islet cells in the pancreas. A diagnosis of MEN1 syndrome is made when tumors are found in two of these glands or organs. The prognosis (chance of recovery) is usually good.
    These tumors may make extra hormones and cause certain signs or symptoms of disease. The signs and symptoms depend on the type of hormone made by the tumor. Sometimes there are no signs or symptoms of cancer.
    The most common condition associated with MEN1 syndrome is hyperparathyroidism. Signs and symptoms of hyperparathyroidism (too much parathyroid hormone) include the following:
    • Having a kidney stone.
    • Feeling weak or very tired.
    • Bone pain.
    Other conditions associated with MEN1 syndrome and their common signs and symptoms are:
    Malignant tumors of the adrenal glands, bronchi, thymus, fibrous tissue, or fat cells may also occur.
    Children with primary hyperparathyroidism, tumors associated with MEN1 syndrome, or a family history of hypercalcemia or MEN1 syndrome may have genetic testing to check for a mutation (change) in the MEN1 gene. Parents should receive genetic counseling (a discussion with a trained professional about the risk of genetic diseases) before genetic testing is done. Genetic counseling also includes a discussion of the risk of MEN1 syndrome for the child and other family members.
    Children who are diagnosed with MEN1 syndrome are checked for signs of cancer starting at age 5 years and continuing for the rest of their life. Talk to your child's doctor about the tests and procedures needed to check for signs of cancer and how often they should be done.
  • MEN2 syndrome includes two main subgroups: MEN2A and MEN2B.
    • MEN2A syndrome
      MEN2A syndrome is also called Sipple syndrome. A diagnosis of MEN2A syndrome may be made when the patient or the patient's parents, brothers, sisters, or children have two or more of the following:
      • Medullary thyroid cancer (a cancer that forms in parafollicular C cells in the thyroid). Signs and symptoms of medullary thyroid cancer may include:
        • A lump in the throat or neck.
        • Trouble breathing.
        • Trouble swallowing.
        • Hoarseness.
      • Pheochromocytoma (a tumor of the adrenal gland). Signs and symptoms of pheochromocytoma may include:
        • Pain in the abdomen or chest.
        • A strong, fast, or irregular heartbeat.
        • Headache.
        • Heavy sweating for no known reason.
        • Dizziness.
        • Feeling shaky.
        • Being irritable or nervous.
      • Parathyroid gland disease (a benign tumor of the parathyroid gland or increase in the size of the parathyroid gland). Signs and symptoms of parathyroid disease may include:
        • Hypercalcemia.
        • Pain in the abdomen, side, or back that doesn't go away.
        • Pain in the bones.
        • A broken bone.
        • A lump in the neck.
        • Trouble speaking.
        • Trouble swallowing.
      Some medullary thyroid cancers occur along with Hirschsprung disease (chronicconstipation that begins when a child is an infant), which has been found in some families with MEN2A syndrome. Hirschsprung disease may appear before other signs of MEN2A syndrome do. Patients who are diagnosed with Hirschsprung disease should be checked for RET gene changes that are linked to medullary thyroid cancer and MEN2A syndrome.
      Familial medullary carcinoma of the thyroid (FMTC) is a type of MEN2A syndrome that causes medullary thyroid cancer. A diagnosis of FMTC may be made when two or more family members have medullary thyroid cancer and no family members have parathyroid or adrenal gland problems.
    • MEN2B syndrome
      Patients with MEN2B syndrome may have a slender body build with long, thin arms and legs. The lips may appear large and bumpy because of benign tumors in the mucous membranes. MEN2B syndrome may cause the following conditions:
      • Medullary thyroid cancer (fast-growing).
      • Parathyroid hyperplasia.
      • Adenomas.
      • Pheochromocytoma.
      • Nerve cell tumors in the mucous membranes or other places.
Tests used to diagnose and stage MEN syndromes depend on the signs and symptoms and the patient's family history. They may include:
See the General Information section for a description of these tests and procedures.
Other tests and procedures used to diagnose MEN syndromes include the following:
  • Genetic testing: A laboratory test that analyzes cells or tissues to look for changes in a gene, chromosome, or protein. These changes may be a sign of a genetic disease or condition. They may also be linked to an increased risk of developing a specific disease or condition. A sample of blood is checked for the MEN1 gene to diagnose MEN1 syndrome and for the RET gene to diagnose MEN2 syndrome.
  • Blood hormone studies: A procedure in which a blood sample is checked to measure the amounts of certain hormones released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. The blood may also be checked for high levels of the hormone calcitonin or parathyroid hormone (PTH).
  • Thyroid scan: A small amount of a radioactive substance is swallowed or injected. The radioactive material collects in thyroid gland cells. A special camera linked to a computer detects the radiation given off and makes pictures that show how the thyroid looks and functions and whether the cancer has spread beyond the thyroid gland. If the amount of thyroid-stimulating hormone in the child's blood is low, a scan to make images of the thyroid may be done before surgery.
  • Sestamibi scan: A type of radionuclide scan used to find an overactive parathyroid gland. A very small amount of a radioactive substance called technetium 99 is injected into a vein and travels through the bloodstream to the parathyroid gland. The radioactive substance will collect in the overactive gland and show up brightly on a special camera that detects radioactivity.
  • Venous sampling for an overactive parathyroid gland: A procedure in which a sample of blood is taken from veins near the parathyroid glands. The sample is checked to measure the amount of parathyroid hormone released into the blood by each gland. Venous sampling may be done if blood tests show there is an overactive parathyroid gland but imaging tests don’t show which one it is.
  • Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show whether there are islet cell tumors in the pancreas. This procedure is also called octreotide scan and SRS.
  • MIBG scan: A procedure used to find neuroendocrine tumors, such as pheochromocytoma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
  • Twenty-four-hour urine test: A procedure used to diagnose neuroendocrine tumors, such as pheochromocytoma. Urine is collected for 24 hours to measure the amounts of catecholamines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher than normal amounts may be a sign of pheochromocytoma.
  • Pentagastrin stimulation test: A test in which blood samples are checked to measure the amount of calcitonin in the blood. Calcium gluconate and pentagastrin are injected into the blood and then several blood samples are taken over the next 5 minutes. If the level of calcitonin in the blood increases, it may be a sign of medullary thyroid cancer.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
There are several types of MEN syndrome, and each type may need different treatment:
  • Patients with MEN1 syndrome are treated for parathyroid, pancreatic, and pituitary tumors.
  • Patients with MEN1 syndrome and primary hyperparathyroidism may have surgery to remove at least three parathyroid glands and the thymus.
  • Patients with MEN2A syndrome usually have surgery to remove the thyroid by age 5 years or earlier if genetic tests show certain changes in the RET gene. The surgery is done to diagnose cancer or to lessen the chance cancer will form or spread.
  • Infants with MEN2B syndrome may have surgery to remove the thyroid to lessen the chance cancer will form or spread.
  • Children with MEN2B syndrome who have medullary thyroid cancer may be treated with targeted therapy (kinase inhibitor called vandetanib).
Treatment of patients with Hirschsprung disease and certain RET gene changes include the following:
  • Total thyroidectomy to lessen the chance that cancer will form.
Treatment of recurrent MEN syndrome in children may include the following:
  • A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.

Pheochromocytoma and Paraganglioma

Pheochromocytoma and paraganglioma are rare tumors that come from the same type of nerve tissue. Most of these tumors are not cancer.
  • Pheochromocytoma forms in the adrenal glands. There are two adrenal glands, one on top of each kidney in the back of the upper abdomen. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla. Pheochromocytoma is a tumor of the adrenal medulla.
    The adrenal glands make important hormones called catecholamines. Adrenaline(epinephrine) and noradrenaline (norepinephrine) are two types of catecholamines that help control heart rate, blood pressure, blood sugar, and the way the body reacts to stress. Some pheochromocytomas release extra adrenaline and noradrenaline into the blood and cause symptoms.
  • Paraganglioma forms outside the adrenal glands near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body. Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of extra adrenaline and noradrenaline into the blood may cause symptoms.
Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests
Anything that increases your chance of getting a disease is called a risk factor. Having a risk factor doesn't mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Talk with your child’s doctor if you think your child may be at risk.
The risk of pheochromocytoma or paraganglioma is increased by having any of the following inherited syndromes or gene changes:
More than half of the children and adolescents diagnosed with pheochromocytoma or paraganglioma have an inherited syndrome or gene change that increased the risk of cancer. Genetic counseling (a discussion with a trained professional about inherited diseases) and testing is an important part of the treatment plan.
Some tumors do not make extra adrenaline or noradrenaline and do not cause symptoms. These tumors may be found when a lump forms in the neck or when a test or procedure is done for another reason. Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other symptoms may be caused by pheochromocytoma, paraganglioma, or other conditions. Check with your child’s doctor if your child has any of the following:
  • High blood pressure.
  • Headache.
  • Heavy sweating for no known reason.
  • A strong, fast, or irregular heartbeat.
  • Feeling shaky.
  • Being extremely pale.
  • Dizziness.
  • Being irritable or nervous.
These signs and symptoms may come and go but high blood pressure is more likely to occur for long periods of time in young patients. These signs and symptoms may also occur with physical activity, injury, anesthesia, surgery to remove the tumor, eating foods such as chocolate and cheese, or while passing urine (if the tumor is in the bladder).
Tests used to diagnose and stage pheochromocytoma and paraganglioma depend on the signs and symptoms and the patient's family history. They may include:
See the General Information section for a description of these tests and procedures.
Other tests and procedures used to diagnose pheochromocytoma and paraganglioma include the following:
  • Plasma-free metanephrines test: A blood test that measures the amount of metanephrines in the blood. Metanephrines are substances that are made when the body breaks down adrenaline or noradrenaline. Pheochromocytomas and paragangliomas can make large amounts of adrenaline and noradrenaline and cause high levels of metanephrines in both the blood and urine.
  • Blood catecholamine studies: A procedure in which a blood sample is checked to measure the amount of certain catecholamines (adrenaline or noradrenaline) released into the blood. Substances caused by the breakdown of these catecholamines are also measured. An unusual (unusual higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher than normal amounts may be a sign of pheochromocytoma or paraganglioma.
  • Twenty-four-hour urine test: A test in which urine is collected for 24 hours to measure the amounts of catecholamines (adrenaline or noradrenaline) or metanephrines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher than normal amounts may be a sign of pheochromocytoma or paraganglioma.
  • MIBG scan: A procedure used to find neuroendocrine tumors, such as pheochromocytoma and paraganglioma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by ascanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
  • Somatostatin receptor scintigraphy: A type of radionuclide scan that may be used to find tumors. A very small amount of radioactive octreotide (a hormone that attaches to tumors) is injected into a vein and travels through the blood. The radioactive octreotide attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body. This procedure is also called octreotide scan and SRS.
  • Genetic testing: A laboratory test that analyzes cells or tissues to look for changes in a gene, chromosome, or protein. These changes may be a sign of a genetic disease or condition. They may also be linked to an increased risk of developing a specific disease or condition. The following are genes that might be tested for in children with pheochromocytoma or paraganglioma: VHL, NF1, RET, SDHD, SDHB, SDHA, MAX, and TMEM127 genes.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of pheochromocytoma and paraganglioma in children may include the following:
Before surgery, drug therapy with alpha-blockers to control blood pressure and beta-blockers to control heart rate are given. If both adrenal glands are removed, life-long hormone therapy to replace hormones made by the adrenal glands is needed after surgery.
Treatment of recurrent pheochromocytoma and paraganglioma in children may include the following:
  • A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
  • A clinical trial of 131I-MIBG therapy.
  • A clinical trial of targeted therapy with a DNA methyltransferase inhibitor.

Skin Cancer (Melanoma, Squamous Cell Cancer, Basal Cell Cancer)

Skin cancer is a disease in which malignant (cancer) cells form in the tissues of the skin. The skin is the body’s largest organ. It protects against heat, sunlight, injury, and infection. Skin also helps control body temperature and stores water, fat, and vitamin D. The skin has several layers, but the two main layers are the epidermis (upper or outer layer) and the dermis (lower or inner layer). Skin cancer begins in the epidermis, which is made up of three kinds of cells:
  • Melanocytes: Found in the lower part of the epidermis, these cells make melanin, the pigment that gives skin its natural color. When skin is exposed to the sun, melanocytes make more pigment and cause the skin to darken.
  • Squamous cells: Thin, flat cells that form the top layer of the epidermis.
  • Basal cells: Round cells under the squamous cells.
ENLARGEAnatomy of the skin with melanocytes; drawing shows normal skin anatomy, including the epidermis, dermis, hair follicles, sweat glands, hair shafts, veins, arteries, fatty tissue, nerves, lymph vessels, oil glands, and subcutaneous tissue. The pullout shows a close-up of the squamous cell and basal cell layers of the epidermis above the dermis with blood vessels. Melanin is shown in the cells. A melanocyte is shown in the layer of basal cells at the deepest part of the epidermis.
Anatomy of the skin, showing the epidermis, dermis, and subcutaneous tissue. Melanocytes are in the layer of basal cells at the deepest part of the epidermis.
There are three types of skin cancer:
Melanoma
Even though melanoma is rare, it is the most common skin cancer in children. It occurs more often in adolescents aged 15 to 19 years.
The risk of having melanoma is increased by having the following conditions:
Other risk factors for melanoma in all age groups include:
  • Having a fair complexion, which includes the following:
    • Fair skin that freckles and burns easily, does not tan, or tans poorly.
    • Blue or green or other light-colored eyes.
    • Red or blond hair.
  • Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time.
  • Having several large or many small moles.
  • Having a family history or personal history of unusual moles (atypical nevus syndrome).
  • Having a family history of melanoma.
Signs and symptoms of melanoma include the following:
  • A mole that:
    • changes in size, shape, or color.
    • has irregular edges or borders.
    • is more than one color.
    • is asymmetrical (if the mole is divided in half, the 2 halves are different in size or shape).
    • itches.
    • oozes, bleeds, or is ulcerated (a condition in which the top layer of skin breaks down and the tissue below shows through).
  • Change in pigmented (colored) skin.
  • Satellite moles (new moles that grow near an existing mole).
Tests to diagnose and stage melanoma may include the following:
See the General Information section for a description of these tests and procedures.
Other tests and procedures used to diagnose melanoma include the following:
  • Skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture.
  • Biopsy: All or part of the abnormal-looking growth is cut from the skin and viewed under a microscope by a pathologist to check for cancer cells. There are four main types of skin biopsies:
  • Sentinel lymph node biopsy: The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node in a group of lymph nodes to receive lymphatic drainage from the primary tumor. It is the first lymph node the cancer is likely to spread to from the primary tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. Sometimes, a sentinel lymph node is found in more than one group of nodes.
  • Lymph node dissection: A surgical procedure in which lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. For a regional lymph node dissection, some of the lymph nodes in the tumor area are removed. For a radical lymph node dissection, most or all of the lymph nodes in the tumor area are removed. This procedure is also called a lymphadenectomy.
Treatment of Melanoma
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of melanoma that has not spread to lymph nodes or other parts of the body includes the following:
  • Surgery to remove the tumor and some healthy tissue around it.
Treatment of melanoma that has spread to nearby lymph nodes includes the following:
Treatment of melanoma that has spread beyond the lymph nodes may include the following:
  • Immunotherapy (ipilimumab).
  • A clinical trial of an oral targeted therapy drug (dabrafenib) in children and adolescents.
Treatment of recurrent melanoma in children may include the following:
  • A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
  • A clinical trial of immunotherapy with immune checkpoint inhibitors (pembrolizumab, nivolumab, ipilimumab) in children and adolescents.
See the PDQ summary on adult Melanoma Treatment for more information.
Squamous Cell and Basal Cell Skin Cancer
Nonmelanoma skin cancers (squamous cell and basal cell cancers) are very rare in children and adolescents. The risk of squamous cell or basal cell cancer is increased by the following:
  • Being exposed to natural sunlight or artificial sunlight (such as from tanning beds) over long periods of time.
  • Having a fair complexion, which includes the following:
    • Fair skin that freckles and burns easily, does not tan, or tans poorly.
    • Blue or green or other light-colored eyes.
    • Red or blond hair.
  • Having actinic keratosis.
  • Having Gorlin syndrome.
  • Past treatment with radiation.
  • Having a weakened immune system.
Signs of squamous cell and basal cell skin cancer include the following:
  • A sore that does not heal.
  • Areas of the skin that are:
    • Small, raised, smooth, shiny, and waxy.
    • Small, raised, and red or reddish-brown.
    • Flat, rough, red or brown, and scaly.
    • Scaly, bleeding, or crusty.
    • Similar to a scar and firm.
Tests to diagnose squamous cell and basal cell skin cancer include the following:
  • Skin exam: A doctor or nurse checks the skin for bumps or spots that look abnormal in color, size, shape, or texture.
  • Biopsy: All or part of a growth that doesn't look normal is cut from the skin and viewed under a microscope by a pathologist to check for signs of cancer. There are three main types of skin biopsies:
    • Shave biopsy: A sterile razor blade is used to “shave off” the growth that does not look normal.
    • Punch biopsy: A special instrument called a punch or a trephine is used to remove a circle of tissue from the growth that does not look normal.
    • Incisional biopsy: A scalpel is used to remove part of an abnormal-looking growth.
    • Excisional biopsy: A scalpel is used to remove the entire growth.
Treatment of Squamous Cell and Basal Cell Skin Cancer
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of squamous cell and basal cell cancer in children may include the following:
  • Surgery to remove the tumor. This may include Mohs micrographic surgery.
    Mohs micrographic surgery is a type of surgery used for skin cancers. The tumor is cut from the skin in thin layers. During surgery, the edges of the tumor and each layer of tumor removed are viewed through a microscope to check for cancer cells. Layers continue to be removed until no more cancer cells are seen. This type of surgery removes as little normal tissue as possible and is often used to remove skin cancer on the face.
Treatment of recurrent squamous cell and basal cell cancer in children may include the following:
  • A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Skin Cancer Treatment for more information.

Intraocular (Uveal) Melanoma

Intraocular melanoma begins in the middle of three layers of the wall of the eye. The outer layer includes the white sclera (the "white of the eye") and the clear cornea at the front of the eye. The inner layer has a lining of nerve tissue, called the retina, which senses light and sends images along the optic nerve to the brain. The middle layer, where intraocular melanoma forms, is called the uvea or uveal tract, and has three main parts: the iris, the ciliary body, and the choroid.
ENLARGEEye anatomy; two-panel drawing shows the outside and inside of the eye. The top panel shows outside of the eye including the eyelid, pupil, sclera, and iris; the bottom panel shows inside of the eye including the cornea, lens, ciliary body, retina, choroid, optic nerve, and vitreous humor.
Anatomy of the eye, showing the outside and inside of the eye including the sclera, cornea, iris, ciliary body, choroid, retina, vitreous humor, and optic nerve. The vitreous humor is a liquid that fills the center of the eye.
Risk Factors
The risk of intraocular melanoma is increased by any of the following:
Tests to diagnose and stage intraocular melanoma may include the following:
Other tests and procedures used to diagnose intraocular melanoma include the following:
  • Fluorescein angiography: A test used to take pictures of the retina in the eye. A yellow dye is injected into a vein and travels throughout the body including the blood vesselsin the eye. The yellow dye causes the vessels in the eye to fluoresce when a picture is taken.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of intraocular melanoma in children is like treatment for adults and may include the following:
Treatment of recurrent intraocular melanoma in children may include the following:
  • A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Intraocular (Uveal) Melanoma Treatment for more information.

Chordoma

Chordoma is a very rare type of slow-growing bone tumor that forms anywhere along the spine from the base of the skull (a bone called the clivus) to the tailbone. In children and adolescents, chordomas form most often in the bones at the base of the skull or near the tailbone, making them hard to remove completely with surgery.
Childhood chordoma is linked to the condition tuberous sclerosis, a genetic disorder in which tumors that are benign (not cancer) form in the kidneys, brain, eyes, heart, lungs, and skin.
Signs and Symptoms
Signs and symptoms of chordoma depend on where the tumor forms. Chordoma may cause any of the following signs and symptoms. Check with your child’s doctor if your child has any of the following:
  • Headache.
  • Double vision.
  • Blocked or stuffy nose.
  • Trouble speaking.
  • Trouble swallowing.
  • Neck or back pain.
  • Pain down the back of the legs.
  • Numbness, tingling, or weakness of the arms and legs.
  • A change in bowel or bladder habits.
Other conditions that are not chordoma may cause these same signs and symptoms.
Tests to diagnose chordoma or to see if it has spread include the following:
Chordomas may recur (come back), usually in the same place, but sometimes they recur in other areas of bone or in the lungs.
Prognosis
The prognosis (chance of recovery) depends on the following:
  • The child's age.
  • Where the tumor forms along the spine.
  • How the tumor responds to treatment.
  • Whether there were changes in bowel or bladder habits at diagnosis.
  • Whether the tumor has just been diagnosed or has recurred (come back).
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment of chordoma in children may include the following:
Treatment of recurrent chordoma in children may include the following:
  • A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change. Patients with changes in the SMARCB1 gene may be treated with tazemetostat in this clinical trial.

Cancer of Unknown Primary Site

Cancer of unknown primary is a disease in which malignant (cancer) cells are found in the body but the place the cancer began is not known. Cancer can form in any tissue of the body. The primary cancer (the cancer that first formed) can spread to other parts of the body. This process is called metastasis. Cancer cells usually look like the cells in the type of tissue in which the cancer began. For example, breast cancer cells may spread to the lung. Because the cancer began in the breast, the cancer cells in the lung look like breast cancer cells.
Sometimes doctors find where the cancer has spread but cannot find where in the body the cancer first began to grow. This type of cancer is called a cancer of unknown primary or occult primary tumor.
ENLARGECarcinoma of unknown primary; drawing shows a primary tumor that has spread from an unknown site to other parts of the body (the lung and the brain). An inset shows cancer cells spreading from the primary cancer, through the blood and lymph systems, to another part of the body where a metastatic tumor has formed.
In carcinoma of unknown primary, cancer cells have spread in the body but the place where the primary cancer began is unknown.
Tests are done to find where the primary cancer began and to get information about where the cancer has spread. When tests are able to find the primary cancer, the cancer is no longer a cancer of unknown primary and treatment is based on the type of primary cancer.
Because the place where the cancer started is not known, many different tests and procedures, including gene expression profiling and gene testing, may be needed to find out what type of cancer it is. If tests show there may be cancer, a biopsy is done. A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist. The pathologist views the tissue to look for cancer cells and to find out the type of cancer. The type of biopsy that is done depends on the part of the body being tested for cancer. One of the following types of biopsies may be used:
When the type of cancer cells or tissue removed is different from the type of cancer cells expected to be found, a diagnosis of cancer of unknown primary may be made. The cells in the body have a certain look that depends on the type of tissue they come from. For example, a sample of cancer tissue taken from the breast is expected to be made up of breast cells. However, if the sample of tissue is a different type of cell (not made up of breast cells), it is likely that the cells have spread to the breast from another part of the body.
When it is not known where the cancer first formed at diagnosis, adenocarcinomas, melanomas, and embryonal tumors (such as rhabdomyosarcoma or neuroblastoma) are tumor types that are often later diagnosed in children and adolescents.
Treatment
For information about the treatments listed below, see the Treatment Option Overviewsection.
Treatment depends on what the cancer cells look like under a microscope, the patient's age, signs and symptoms, and where the cancer has spread in the body. Treatment is usually the following:
Treatment of recurrent cancer of unknown primary in children may include the following:
  • A clinical trial that checks a sample of the patient's tumor for certain gene changes. The type of targeted therapy that will be given to the patient depends on the type of gene change.
See the PDQ summary on adult Carcinoma of Unknown Primary for more information.

To Learn More About Childhood Cancer

For more information from the National Cancer Institute about unusual cancers of childhood, see the following:
For more childhood cancer information and other general cancer resources, see the following:

About This PDQ Summary

About PDQ

Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.

Purpose of This Summary

This PDQ cancer information summary has current information about the treatment of unusual cancers of childhood. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

Reviewers and Updates

Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.

Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).

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The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Unusual Cancers of Childhood Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/childhood-cancers/patient/unusual-cancers-childhood-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389276]
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The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.

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